ICARS consists of a one-hundred-point semi-quantitative scale based upon 19 simple testing manoeuvres compartmentalized into postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders and has been previously used in this patient population with good inter-rater reliability.

Scores for each subscale quantify the extent of ataxia in each clinically important area. Subscale scores are summed to give a total score ranging from 0 (best) to 100 (worst).

Treatment with idebenone, Coenzyme Q10 or vitamin E (if taken at a dose 5 times above the daily requirement) within the past month

Patients with International Cooperative Ataxia Rating Scale (ICARS) score of greater than 54 or less than 10 at screening

Pregnancy and/or breast-feeding

Clinically significant abnormalities of clinical hematology or biochemistry including, but not limited to, elevations greater than 1.5 times the upper limit of normal of Aspartate Aminotransferase (AST; also known as GOT), Alanine Aminotransferase (ALT; also known as GPT) or creatinine

History of abuse of drugs or alcohol

Participation in the previous (Phase II) study of idebenone at the National Institutes of Health (NIH)

Participation in a trial of another investigational drug within the last 3 months

Gender

Both

Ages

8 Years to 17 Years

Accepts Healthy Volunteers

No

Contacts ICMJE

Contact information is only displayed when the study is recruiting subjects